A glomus tumor of the middle ear

Glomus tumor is one of the varieties of paraganglia, which are clusters of hormonally active and receptor cells that have a common origin with the ANS. A distinction is made between chromaffin (i.e., bound by chromic acid salts) and non-chromaffin paraganglia. The former were previously grouped under the name "adrenal system"; they are functionally associated with the sympathetic division of the ANS, and the latter with its parasympathetic division.

The largest paraganglia are the adrenal (adrenal medulla) and lumbar aortic. There are also laryngeal, tympanic, jugular and other paraganglia. Paraganglia include clusters of chromaffin cells in the form of glomuses (nodes), including carotid, supracardiac and other localizations. In macrostructure, individual glomus tumors are clusters of arteriovenous anastomoses surrounded by a connective tissue capsule and divided into lobules and cords. A large number of small granules containing adrenaline or noradrenaline are scattered in the cytoplasm of chromaffin cells. In non-chromaffin cells, secretion of polypeptide hormones that are not catecholamines is assumed. In glomus tumors, the vascular network is well developed; most secretory cells are adjacent to the walls of vessels. The centrifugal processes of the cells of the lateral horns of the gray matter of the spinal cord and the vegetative nuclei of the glossopharyngeal and vagus nerves end on the cells of the paraganglia. The nerve fibers penetrating the paraganglia end in chemoreceptors that perceive changes in the chemical composition of tissue and blood. A particularly important role in chemoreception belongs to the carotid glomus, located in the area where the common carotid artery divides into the internal and external. Paraganglia are sometimes the sources of tumor development - paraganglia and chromaffin - or systemic diseases such as Barre-Masson disease (syndrome), which is a manifestation of the so-called active glomus tumors in the circulatory system, which produce certain substances that cause such general symptoms as asthma attacks, tachycardia, arterial hypertension, increased body temperature, dry skin, dysmenorrhea, insomnia, a feeling of fear and other phenomena of psychoemotional dysfunction, indicating the influence of these substances on the limbic-reticular system of the brain. Many of these signs are characteristic of a glomus tumor of the middle ear.

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What causes glomus tumor of the middle ear?

Normally, the jugular glomus is a kind of arteriovenous shunt located in the area of the bulb of the jugular vein at the jugular foramen of the base of the skull (posterior lacerated foramen), consisting of vascular arteriovenous plexuses. As an independent nosological form, the glomus tumor of the jugular paraganglia was formed in 1945. Despite the fact that in structure this tumor belongs to benign neoplasms with slow development, it can pose a significant danger in relation to the destruction that it can cause to neighboring vital organs during its spread. This destructive effect is caused not only by the pressure of the tumor itself, but also by those still not fully studied "caustic" substances that are released on its surface and cause resorption with tissues in contact with them. Primarily located in the area of the bulbi venae jugularis, the tumor can spread in three directions in its development, causing the emergence of three syndromes corresponding to each of the directions shown in the figure.

Symptoms of Glomus Tumor of the Middle Ear

Otiatric syndrome debuts with the appearance of a pulsating blowing noise in one ear, changing in intensity or disappearing when the common carotid artery is compressed on the corresponding side. The rhythm of the noise is synchronized with the pulse rate. Then progressive unilateral hearing loss occurs, first of a conductive nature, and in case of tumor invasion in the inner ear - of a perceptual nature. In the latter case, vestibular crises of increasing intensity also occur, usually ending in the shutdown of both vestibular and auditory functions on the same side. Objectively, signs of the presence of blood or a vascular tumor in the tympanic cavity are noted, which shines through the eardrum as a reddish-pink or bluish formation, often pushing the eardrum outward. Further development of the tumor leads to destruction of the eardrum and the exit of tumor masses in the external auditory canal, red-bluish in color, easily bleeding when probing with a button probe.

Otoscopy reveals a cyanotic tumor of fleshy consistency, occupying the entire bone part (and more) of the external auditory canal, dense and bleeding, pulsating and growing into the defect of the posterosuperior part of the lateral wall of the epitympanic space, "bathing" in abundant purulent discharge. The tumor can penetrate through the aditus ad antrum into the cells of the mastoid process or, when spreading forward, into the auditory tube and through it into the nasopharynx, imitating the primary tumor of this cavity.

The neurological syndrome is caused by the growth of paraganglioma in the posterior cranial fossa, where it causes damage to the IX, X, XI cranial nerves, causing the development of the lacerated foramen syndrome, through which these nerves leave the cranial cavity, manifested by signs of their damage: paresis or paralysis of the tongue on the corresponding side, articulation disorder, open nasal speech and liquid food entering the nose (soft palate paralysis), swallowing disorder, hoarseness, aphonia. With this syndrome, otitis symptoms are absent or insignificant. In case of further progression of the process, the tumor can penetrate into the lateral cistern of the brain and cause MMU syndrome with damage to the facial, vestibulocochlear and trigeminal nerves. The spread of the tumor in the brain can initiate the development of such syndromes as Berne's and Sicard's syndromes.

Berne's syndrome is an alternating paralysis that develops as a result of damage to the pyramidal tract in the medulla oblongata and is manifested by contralateral spastic hemiparesis, homolateral paralysis of the palate and swallowing muscles with sensory disturbances in the posterior third of the tongue, as well as homolateral paralysis of the accessory nerve (paresis or paralysis of the sternocleidomastoid and trapezius muscles): difficulty turning and tilting the head to the healthy side, the shoulder on the affected side is lowered, the lower angle of the scapula deviates from the spine outward and upward, and shrugging the shoulders is difficult.

Star's syndrome is manifested by neuralgia of the glossopharyngeal nerve: sudden unbearable stabbing pain on one side of the soft palate during the consumption of hard, especially hot or cold, food, as well as during chewing, yawning and speaking in a loud voice; the attack of pain lasts about 2 minutes; the pain radiates to the tongue, jaws, adjacent part of the neck and ear.

Neurological syndrome may manifest itself with signs of increased intracranial pressure (congestion of the optic nerve discs, persistent headaches, nausea, vomiting).

Cervical syndrome is similar in manifestation to an aneurysm of a large cervical vessel and is caused by the presence of a pulsating tumor in the lateral part of the neck.

The clinical course and symptoms of glomus tumor of the middle ear are characterized by a long and slow development over many years, which in untreated cases goes through otitis, neurological (damage to peripheral nerves), cervical, intracranial and terminal phases, growing into the surrounding large veins and spaces of the middle ear.

Diagnosis of glomus tumor of the middle ear

Diagnosis of glomus tumor of the middle ear is difficult only in the initial stages of the disease, however, pulsating noise, one of the very first signs of glomus tumor of the middle ear, which occurs even before the formation penetrates into its cavity, should always alert the doctor regarding the presence of this disease, and not only explain this noise by some defect of the carotid artery, for example, narrowing of its lumen by an atherosclerotic process. Of significant importance in diagnosis are the consistently occurring conductive hearing loss, signs of cochlear and vestibular dysfunction, lacerated foramen syndrome, cervical pseudoaneurysmal symptoms, as well as the picture described above during otoscopy. Diagnostic methods are completed by radiography of the temporal bones in the projections according to Schüller, Stenvers, Shosse III and II, in which the radiographs can visualize the destruction of the tympanic cavity and epitympanic space, the expansion of the lacerated opening and the lumen of the bony part of the external auditory canal.

Histological examination reveals closely adjacent polygonal giant cells with nuclei of different shapes and cavernous tissue.

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Treatment of glomus tumor of the middle ear

Treatment of glomus tumor of the middle ear includes both surgical removal of the tumor and physiotherapeutic methods (diathermocoagulation, laser evaporation of tumor tissue followed by radio- or cobalt therapy). Surgical intervention should be performed as early as possible and in the widest possible range, like petromastoid surgery.

What is the prognosis for glomus tumor of the middle ear?

Depending on the time of diagnosis, the direction of tumor growth, its size and the treatment, glomus tumor of the middle ear has a cautious to very serious prognosis. Relapses are very common.